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阵发性冷性血红蛋白尿患者外周血涂片中性粒细胞、单核细胞吞噬红细胞现象1例

2020-06-13 郭明卫 李妤蓉 国际检验医学杂志

骨髓形态学检验中发生吞噬血细胞的原因很多, 且被吞噬的细胞类 型也较多。但外周血出现 中 性粒 细胞、单核细胞吞噬红细胞是非常罕见的现象。在 临 床工作中,笔者发现1例外周血 中 性粒细胞、单核细 胞

骨髓形态学检验中发生吞噬血细胞的原因很多, 且被吞噬的细胞类 型也较多。但外周血出现 中 性粒 细胞、单核细胞吞噬红细胞是非常罕见的现象。在 临 床工作中,笔者发现1例外周血 中 性粒细胞、单核细 胞吞噬红细胞的现象,现报道如下。

1临床资料   

患者,女,78岁,农民。主诉:间断发热1个月,加 重肢体抽动1d入沙河市人民医院。患者于1个月前 无明显诱因出现间 断发热,体温38.0 ℃,无明显咳嗽 咳痰、胸痛,无意识障 碍,反复发作,曾在当地输液治 疗,症状无明显好转,1d前患者出现肢体抽动明显, 仍有发热,体温在38.5℃左右,问话不答,曾有小便失 禁、纳差、乏力,为进一步诊治于2019年4月11日入 沙河市人民医院。CT检查:头颅平扫老年脑,肺部平 扫右肺上叶小结节,纵膈、腋窝肿大淋巴结。双侧胸 腔少量积液。上下腹脾CT平扫:脾略大,结肠扩张积 气,胆囊大。B超显示:二尖瓣关闭不全(轻),三尖瓣 关闭不全(轻+),双肾皮质回声增强。胆囊大于正 常。患者考虑诊断为:( 1)多脏器衰竭;( 2)肺部感染; ( 3)高血压3级,高危;( 4)电解质紊乱,低钠血症,低 氯血症;( 5)肝功能异 常;( 6)肾功能异 常;( 7)低蛋 白 血症;( 8)帕金森病。入院后给予吸氧,CCJ监护,给 予哌拉西林他唑巴坦、热毒宁、还原型谷胱甘肽以抗 感染、保肝、支持对症等综合治疗,住院1d,患者病情 危重,应继续住院治疗,向其讲明出院有可能随时出 现死亡,但劝说无效,患者家属坚持要求出院。

2实验室检查   

白细胞21.83×10 9/L,红细胞2.74×1012/L,血红 蛋白89g /L,中性粒细胞百分比84.90%,血小板160× 10 9/L,网织红细胞0.006,钾离子3.94mmol /L,钠离子 125.3mmol /L,氯离子 86.60 mmol /L,钙 离子 2.30 mmol /L,总蛋 白67.34g/L,清蛋 白26.19g/L,球蛋 白48.29g/L,清蛋白与球蛋白比值为0.54,总胆红素 53.49μmol/L,直接胆红素21.41μmol /L,间接胆红 素32.08μmol /L,丙氨酸氨基转移酶17.85U/L,天门 冬氨酸氨基转移酶216.50U/L,谷氨酸氨基转移酶 41.60U/L,碱性磷酸酶 170.13 U/L,尿素氮 14.58 mmol /L,肌酐251.11μmol /L,自身抗体(抗核抗体、抗 U1RBNP/抗Sm、抗Sm、抗 SSA、SSB、抗 Sc1 - 70、 抗Jo - 1、抗核糖体 P蛋 白)阴性,梅毒螺旋体(梅毒螺 旋体特异性抗体定性试验、梅毒甲苯胺红不加热试验 半定量)均阴性,抗人球蛋 白检测卡检测直抗试验和 游离、放散试验均为阳性。血细胞计数结果触发血液 分析仪复检规则,遂进行血涂片染色镜检可见中性粒细 胞吞噬红细胞现象(图1A~C)和单核细胞吞噬红细胞 现象(图1D)。并可见红细胞大小不一或成锯齿状红细 胞,球形红细胞。


注: A~C表示中 性粒细胞吞噬红细胞现象; D表示单核细胞吞噬 红细胞现象。 图1  血涂片染色镜检结果(4×100)

3讨  论   

本例患者表现出贫 血、黄疸、脾略大等阳性 体征, 血清外观为褐红色,尿液为酱油色,抗人球蛋白检测直 抗试验和游离、放散试验均为阳性,提示患者可能为自 身免疫性溶血性贫血(AIHA)。血清总胆红素、直接胆 红素、间接胆红素是判断高胆红素血症的检测指标,虽 根据《诊断学》第8版提供的溶血 性黄疸时,直接胆红 素/间接胆 红 素 比 例 <20%,总 胆 红 素 通 常 <85.00 μmol /L,但根据文献[ 1]支持本 例患者 为 AIHA;成熟 红细胞大小和形态变化也支持 AIHA的诊断。 AIHA 是由红细胞膜组分 的自身抗体引起的获 得性溶血性贫血,按抗体反应最适温度分为温抗体型 和冷抗体型,后者又分为冷凝集综合征和阵发性冷性 血红蛋白尿(PCH)两 类。根据文献报 道[ 2 -3],本病例 最可能的诊断为PCH。 PCH 是 AIHA 中一种罕见的冷反应性自身抗体引起的免疫性溶血性贫血,以全身或局部受寒后突然 发生大量血管内溶血和血红蛋 白尿为特征。中 性粒 细胞吞噬红细胞已被报 道 为 PCH 的突出特征[ 4],有 研究阐明中 性粒细胞和单核细胞吞噬红细胞的免疫 机理,免疫相关的红细胞被吞噬最常见的是PCH[ 3 -5]。 需要指出的是,多数文献都提到在溶血 性 贫 血时 患者的网织红细胞增加,作为溶血 性 贫 血 诊 断 的检验 项目之一。而本例患者网织红细胞百分比正常,其原 因可能为慢性肾病所致[ 6]。本 例患者尿素氮和肌酐 均增高,提示慢性肾病累积对患者的肾脏产生实质性 损害,导致患者 体内红细胞生成素生成减低,网织红 细胞升高不明显或不升高。 据文献报 道,观察中 性粒细胞吞噬红细胞现象能 提供PCH 的诊断线索及提高诊断的准确性[ 5]。同时, 建议在进行血常规检测时,不要依赖血液分析仪,在检 测结果触发复检规则时,应及时进行血涂片检查,防止 漏诊和误诊,提高血常规检验的准确率和符合率。

参考文献略。

原始出处:

郭明卫,李妤蓉,阵发性冷性血红蛋白尿患者外周血涂片中性粒细胞、 单核细胞吞噬红细胞现象1例[J],国际检验医学杂志,2020,41(2).

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